One of the more common reasons for women between puberty and the menopause to have vulval symptoms is infection and in particular due to the yeast Candida albicans. This occurs during pregnancy, while using oral contraceptives and following prolonged courses of broad-spectrum antibiotics.

It is commonly stated that the postmenopausal vulva is more vulnerable to infection, but on the basis of alterations within the vagina. Studies using swab and culture methods are now being replaced by molecular techniques for ‘microbial communities’, but little is available for description of what happens after the menopause [28].

It is most unlikely that postmenopausal women will have symptoms, e.g. chronic itch, vaginal discharge and dyspareunia due to vaginal yeasts, unless they are taking topical or systemic estrogen therapy [29] or are diabetic (Fig. 20.2). Before ascribing vulval symptoms in any postmenopausal women to yeasts, a low vaginal swab should be taken (by the woman when she is symptomatic or by her doctor) and examined for confirmation. Symptoms thought to be due to yeasts but associated with a negative swab require closer examination (as per the next section). Those women who have confirmed yeasts and are not on estrogen therapy should be screened/tested for diabetes mellitus.

The reasons for younger women being more susceptible to Candida species remain unclear, but include promotional changes in pH, direct effect of estrogens on adherence of yeasts to vaginal mucosa, effects of menstruation and tampon/menstrual pad use on vaginal microflora [30], an estrogen effect on vaginal immunity [31] and the availability of antifungal therapies as over-the-counter sales without an examination or prescription from their doctor [32].

The impact of bacterial vaginosis and chlamydial or gonococcal bartholinitis is less likely in postmenopausal women. Viral changes associated with human papilloma virus will be discussed later. Herpes simplex lesions may still occur or reactivate in postmenopausal women. Rarely herpes zoster, due to varicella-zoster virus, can be seen involving part of the vulval area, i.e. unilateral involvement of thoracolumbar T12/L1 or sacral S2, S3 or S4 dermatomes (Fig. 20.3). Molluscum contagiosum has been seen in immune-competent postmenopausal women although more commonly seen in young adults (Fig. 20.4).

Superficial skin infections due to staphylococcal or streptococcal species are less frequently seen than in premenopausal women (partly associated with hair removal by waxing or shaving), but may follow skin damage from urinary or faecal incontinence (see section below on vulval dermatitis).

Deeper infections, e.g. Hidradenitis suppurativa (Fig. 20.5), do occur in perimenopausal women but usually settle after the menopause [33]. Skin infection complicating vulval surgery can complicate healing in any age group, particularly if obese, diabetic or immune-compromised. Necrotizing fasciitis or Fournier’s gangrene is reported but extremely uncommon [34].

The majority of patients who attend a gynaecology, dermatology or genitourinary clinic do so because they are symptomatic. For most, they will complain of itch or scratching (pruritus), while a minority will have pain, burning, soreness or rawness. Some will attend because of splitting, discomfort or sexual difficulty. However, some patients will be referred because abnormal, unusual or altered appearances will be noted when the patient attends for something else, e.g. to have a smear taken, or a gynaecological examination for other reasons.

While clinical appearances may allow the medical or nursing practitioner to make a diagnosis, more frequently it will require an appropriate history and to use correct examination techniques and biopsy procedures. History should include past history, e.g. flexural eczema or asthma, and family history, e.g. psoriasis and LS both having familial clustering. Other autoimmune disorders may be relevant. Careful documentation of all treatments previously used including topical applications is important to recognise that the appearances may have been modified, to avoid prescribing something that has not worked previously, and to identify possible contact allergens or irritants including soaps, perfumes, preservatives and components of sanitary pads.

Examination should include the fingernails (Fig. 20.6) and skin of the hands, wrists, elbows, scalp and face; the oral cavity (Fig. 20.7), gums and tongue; the trunk and the lower limbs. Examination of the vulva requires exposure of the pudendum from pubes and inguinal areas to the natal cleft and cannot be done easily with the patient lying flat on a couch; the dorsal position gives some viewing but the patient then needs turning on her side, sometimes with straightening of the upper leg, to get complete viewing. Adequate lighting is paramount. Colposcopy is not essential, but a colposcopy chair provides good positioning, and the associated light of the colposcope allows viewing without the examiner’s head or hair obscuring the light source. Areas of colour change are noted and examined at higher magnification (a hand-held looking glass is an alternative to a colposcope and may now come with an in-built light source) for change in surface contour – exophytic or ulcerative. Red areas may represent altered capillary dilatation, but angiogenesis should be considered. White areas with abnormally thickened keratin may be the result of scratch damage but may represent more significant pathology.

Applications of 5 % acetic acid or 1 % toluidine blue solutions are not performed for every patient but certainly for anyone who has previously had an abnormal smear. Acetic acid penetrates mucosa quickly but not keratinised skin; it precipitates nucleoprotein so that neoplastic areas become visible (as for colposcopy of the cervix [35]), but it will take longer, the acetowhite epithelium may not be as obvious as on the cervix, and little information can be obtained if scratching has produced thickening of the keratin (hyperkeratosis or lichenification). Acetowhite epithelium does not equate to VIN or to HPV presence [36], and biopsy is required of the area. Toluidine blue stains the nuclei of cells (as haematoxylin does in histopathology). Surface squames consist mainly of cytoplasm with small pyknotic nuclei and therefore take up minimal blue staining. Where squamous differentiation and maturation is abnormal (as for VIN, usual type) the nuclei are larger, or if the surface cells have been scratched or scraped away, blue areas of staining will be defined, and these should be biopsied.

Biopsy is not necessary for every diagnosis. Some lesions, e.g. dermatitis/eczema, will be characteristic in their distribution, e.g. interlabial folds or perianal skin. Other lesions may have non-specific appearances as described above, and biopsy will be the only way to characterise the lesion (see classification below). Local anaesthesia, e.g. 2 % lignocaine with adrenaline as a vasoconstrictor, is infiltrated via a 27-gauge needle adjacent to but not into the lesion, and the biopsy is taken with a 4 mm biopsy (e.g. Stiefel) punch. Haemostasis is obtained with silver nitrate or Monsel’s solution and pressure for a few minutes. If the patient is taking aspirin or warfarin, or multiple sites need to be biopsied to map out the extent of the lesion, e.g. Paget’s or VIN, the biopsies are better taken in the theatre, often with a short general anaesthetic and the biopsy sites undersewn with sutures for haemostasis [37].

Contact dermatitis occurs as an allergic response to various allergens including topical antibiotics, anaesthetic and antihistamine creams, deodorants and perfumes (e.g. Balsam of Peru as used in various haemorrhoid creams), lanolin, azodyes in nylons, biological washing powders, etc. An increasing number of referrals to vulval clinics are being seen because of the current trend to remove vulval hair. Some women have applied depilatory creams or waxes with subsequent irritant reaction, while others have inflicted skin damage by shaving.

An increasing challenge in the elderly is control of bladder and bowel function. Sometimes the vulval skin will be irritated by the constant wearing of pads or incontinence devices, but also by the effect of urine or faecal material in contact with the skin. Such events do not necessarily relate to the menopause, although researchers have highlighted the role estrogen plays in maintaining function of the lower urinary tract which in turn preserves urinary continence. As well as the genital tract, the urethra, bladder and pelvic floor also have estrogen receptors. Postmenopausal low levels of estrogen lead to urogenital atrophy and are associated with lower urinary tract symptoms, such as frequency, urgency, incontinence and nocturia [39]. The diminished action of estrogen reduces sensitivity of the urethral smooth muscle, which predisposes to incontinence. The prevalence of urge incontinence, in particular, increases with the menopause [40]. Elderly women have increased susceptibility to the so-termed incontinence dermatitis when the perineal skin is subject to urinary moisture under occlusion. The skin becomes inflamed and its barrier function is damaged by friction, rise in pH and faecal enzymes; this damage is perpetuated by chronic incontinence [41].

The clinical appearance of dermatitis (Fig. 20.8) is diffuse but sometimes focal erythema and oedema with superimposed infection or lichenification (Fig. 20.9). The terms ‘lichen’ and ‘lichenification’ cause confusion because of their similarity. Lichenification describes the patchwork covering of the rocks of the West of Scotland (and elsewhere) and is best illustrated by the appearance of the skin over the elbows and knuckles (metacarpophalangeal joints) where the shin is thickened and patterned unlike skin elsewhere. Biopsy is usually not required unless there is no response to treatment; then other diagnoses should be considered.

Patch testing may identify the allergen to allow removal or avoidance of the factor, and moisturising cream or mild steroids should provide local control. Haverhoek et al. [42] reported that 81 % of the patients that they saw with vulval pruritus had at least one contact allergen detected on patch `testing.

Some topical preparations will cause irritation over time (i.e. in a chronic fashion). The vulval appearances will be less obvious, with minimal erythema but with pallor or pigmentation and with dryness, thickening and cracking – lichenification or lichen simplex chronicus. Lichen simplex chronicus (previously known as ‘neurodermatitis’) occurs in normal skin which becomes thick and fissured in response to the trauma of constant scratching. These lesions are not usually symmetrical and occur in vulval areas accessible to scratching.

Treatment consists of the use of emollients or topical corticosteroids of low-to-moderate potency. Sometimes, sedation at night is useful to stop nocturnal scratching. Once control is gained, assessment for an underlying cause or lesion is often necessary.

Lichen sclerosus represents approximately one quarter of the patients attending our vulval clinic, and the majority of them will be postmenopausal [43]. It is found once in every 800 prepubertal girls [44] and increases with age to be reported as frequent as 1 in 30 elderly women in residential care [45]. It is an inflammatory dermatosis and is frequently symptomatic, causing pruritus, sleeplessness, dyspareunia and constipation. It may produce major architectural changes and alterations in vulval appearance and function and has a small but important risk of cancer; in the UK 60–70 % of vulval squamous cell carcinomas occur against a background of LS [46].

Lichen sclerosus involves the pudendum either partially or completely as a ‘figure of eight’, encircling the vulva and anus and altering the clitoris and its prepuce, the labia minora and inner aspects of the labia majora and the fourchette and perineum. It is usually symmetrical, bilateral and central, but may involve more lateral structures including the genitofemoral creases and the natal cleft. It involves the skin, but does not involve the mucosa of the vestibule, vagina or anus (although ‘overlap’ with erosive lichen planus is recognised). The lesions consist of thin, pearly, ivory or porcelain white crinkly plaques. Sometimes there is shrinkage and absorption of the labia minora, but sometimes they coapt anteriorly to cause clitoral phimosis and narrowing of the introitus to make intercourse impossible and to impede micturition. Scratching may result in epidermal thickening (lichenification) but sometimes erosion and superficial ulceration (the previous term used by clinicians and endorsed by the ISSVD was ‘lichen sclerosus et atrophicus’). Areas of ecchymoses and later haemosiderin pigmentation are common. LS may involve the trunk and limbs in up to 20 % of patients [47] (Fig. 20.10).

The histological features of LS include epidermal atrophy, dermal oedema, hyalinisation of the superficial dermal collagen and subdermal chronic inflammatory infiltrate. However, occasionally the clinical features will resemble typical LS, and yet the biopsy shows minimal features. Sometimes the terms ‘early’ and ‘late’ are used to indicate the level of the inflammatory process, and these must not be misinterpreted as recent compared to longer duration.

The cause(s) of LS must consider the increased frequency in women (c.f., men) and with increasing age. It may show clustering within families, and a link with HLA DQ7 has been reported [48]. The association with autoimmunity has been widely reported. In the last decade Howard et al. [49] have found circulating basement membrane zone antibody in the serum of patients with LS, and Oyama et al. [50] describe an antibody to extracellular matrix protein 1. Farrell et al. [51] described the presence of interferon-gamma and other inflammatory cytokines within lesional epidermis, underlying dermis and within the inflammatory areas.

Explanation is required why LS is frequently seen in the UK, Europe, and especially Scandinavia, North and South America, Oceania and Southern Africa and yet is infrequent in Northern Africa, Arabia, Pakistan, India, Malaysia, Thailand, Singapore, Philippines and Hong Kong. The authors’ observations are offset by data that a quarter of the women attending vulval clinic were born outside the UK, including countries within the areas listed above. It is rare to diagnose LS in women newly arrived in London, but prolonged domicile may be important. The authors are currently researching the possible role of vitamin D deficiency in causing LS. Vitamin D is generated from sun exposure to the head, arms and body and from oily fish and egg yolks within the diet. Data gathered to date include many older women do not leave the UK for holidays in the sun, and avoid sun exposure or use high factor sun block (some of the patients have developed skin cancers in exposed areas and have been warned by their dermatologists to keep out of the sun, plus our latitude of 56 north; pigmented skin in some patients and more protective clothing in some ethnic and religious groups reduce the effect of the sun in producing vitamin D in the skin); avoid eggs because of their concurrent cardiovascular morbidity and therapy to lower cholesterol levels; and have little enthusiasm for eating certain fish (or are vegetarian). A few women take cod liver oil or vitamin supplements, e.g. with calcium, to protect their bones as the current literature discourages the use of hormone replacement therapy. Serum levels of vitamin D are abnormally low in some of our patients, but we are comparing these with control patients because many older people in London, including those who arrive from other countries, may have chronically low vitamin D levels. Certainly vitamin D deficiency, with rickets and osteomalacia, is being reported from British studies. Vitamin D deficiency has been linked with autoimmune diseases and certain cancers. Dietary supplementation may become an easier option than sending every postmenopausal woman for a holiday in the sun.

The treatment of LS usually requires the super-potent topical corticosteroid clobetasol propionate and following the protocols described by the British Association of Dermatologists [52]. A fingertip or ‘pea-sized’ amount of clobetasol ointment (Dermovate) is applied at night for 4 weeks, alternate nights for 4 weeks and then twice a week for 4 weeks before review. The pharmacokinetics of clobetasol suggests one application per 24 h is adequate. Many patients require reassurance that the potent steroid will not damage their skin when used according to instructions and to use applications on a regular basis because stopping treatment will frequently cause a rebound of symptoms. A 30 g tube should last for at least 12 weeks, and most patients with ongoing symptoms require 30–60 g of clobetasol per year. Emollients are used during the day and a soap alternative while washing. Testosterone cream no longer has any role in treating women. Alternative treatment use must be done with careful vigilance because of the increased risk of malignancy in patients who do not respond to clobetasol.

The lesions of lichen planus may be seen on mucous membranes or on cutaneous surfaces. Involvement of the vulva is usually with white patterned areas that are sometimes elevated and thickened (hypertrophic lichen planus) or may appear red and raw with features of erosion. Changes in the mouth are often seen. The vulval lesions may extend into the vagina where scarring, stenosis and adhesions make intercourse painful or impossible. In a small number of cases, ‘crossover’ from LS (involving the vulval skin) to lichen planus (involving the vestibule and vaginal mucosa) occurs (Fig. 20.12).

Histology will show liquefactive degeneration of the basal epidermal layer, long and pointed rete ridges, with parakeratosis, acanthosis and a dense dermal infiltrate of lymphocytes close to the dermal epidermal margin.

When the condition is severe, treatment can be difficult, requiring systemic steroids, azathioprine, hydroxychloroquine or other immune-modifying or chemotherapy agents such as methotrexate or mycophenolate mofetil. Lesser symptoms, particularly those externally on the vulva, can be managed with the application of topical corticosteroids; vaginal lesions can be managed with Colifoam (hydrocortisone) or Predfoam (prednisolone).

Rarely, vulval cancer will arise in association with hypertrophic lichen planus [71, 72].

Although this term sounds closely similar to cervical intraepithelial neoplasia, there are some striking differences. Cervical neoplasia occurs in the transformation zone in association with squamous and columnar epithelium. The cervix has an epithelium where depth of changes can be judged: the vulva is the skin (epidermis) and, in addition to a basal layer which may be convoluted with deep rete ridges, also has a spinous or prickle cell layer, a granular layer and a superficial cornified or keratinised layer. Thus, dividing VIN into VIN 1, VIN 2 and VIN 3 is not easy or reproducible. While CIN 1 is a common biopsy finding and is part of a spectrum with possible progression to CIN 3, VIN 1 is uncommon and does not appear to have neoplastic potential.

The ISSVD Vulval Oncology Subcommittee has made recommendations about the terminology of VIN [61]. They argue that intraepithelial neoplasia of the vulva should be divided into two types: the first, known as VIN of usual (sometimes classic) type, encompasses what was previously VIN 2 and VIN 3 and is almost always associated with oncogenic HPV. These lesions may be pigmented (brown), white (hyperkeratotic) or red in appearance, and the histological features are described as warty, basaloid or mixed – the distinction may not have any prognostic significance, and warty and basaloid features may be seen concurrently in multifocal disease or seen over different examinations. The second is called differentiated VIN, usually seen in older women with LS or squamous cell hyperplasia and not associated with HPV (see above in association with LS) (Fig. 20.13).

Patients with CIN are usually identified by abnormal cervical cytology, whereas women with VIN present with symptoms, including itching, irritation, discomfort or splitting with intercourse in more than 80 %.

We know that when loop excision of the cervix is performed to treat CIN, less than 1 % of specimens will be found with microinvasive carcinoma. However, the chances of finding invasive cancer within the lesions of usual-type VIN range between 15 and 22 %, as summarised by MacLean [54] from data from five publications. Nugent et al. [73] have reported that postmenopausal women with VIN were more likely than premenopausal women to have related carcinoma. The difficulty with recognising invasion is partly because keratinised lesions will not show colposcopically visible features of vessel atypia/abnormality (angiogenesis) as it does for the cervix. The significance of invasion, even as little as 1 mm below the epidermo-dermal junction of the nearest dermal junction of the nearest papilla, is the increased chance of lymph node metastases; this risk only becomes significant with measurements of more than 3–5 mm from the nearest basement membrane of CIN.